Congenital hepatic fibrosis is an autosomic recessive disease, manifested in children or in young adults by hematemesis due to rupture of esophageal varices. Bacterial cholangitis may occur in some patients, Early diagnosis and treatment with portosystemic anastomosis and/or antibiotics may lead to a long survival with a good quality of life Diagnosis requires histologic confirmation normal hepatic lobules are surrounded by broad fibrous bands, and an increased number of bile ducts is usually seen in the portal tracts. Liver function remains well preserved This report consists of 3 patients:a 17-year-old woman, a 10-year-old boy and a 24-year-old woman who developed severe upper GI bleeding due to ruptured esophageal varices. The first two patients were successfully operated on using the Sugiura technique for portal decompression Their current survival after surgery is 3 and 9 years, respectively, with normal physical examination and liver function tests, The third patient died shortly after admission, due to the concurrence of a severe suppurative cholangitis.